Faculty Bibliography

OBJECT: The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation. METHODS: Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise. RESULTS: Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15-120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants. CONCLUSIONS: Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification

This article reviews the current status of cochlear implantation in both adults and children, including expanding candidacy groups, bilateral implantation, advances in speech processing software, internal and external device hardware, surgical techniques and outcomes. Promising advances, novel therapies and evolving concepts are also highlighted in terms of their future impact on clinical outcomes

OBJECTIVES: To determine whether significant differences in cochlear implant (CI) performance exist between older and younger CI recipients. DESIGN: Retrospective comparison of audiometric data. SETTING: University hospital center. PARTICIPANTS: Sixty participants who received CIs at age 70 and older and 48 younger adults implanted between age 18 and 69. MEASUREMENTS: Speech outcome scores (Consonant-Nucleus-Consonant words and phonemes and City University of New York Sentence test in quiet and noise). RESULTS: Older participants show significant improvement in speech perception scores after implantation. Although on average they do not perform as well as younger adults, this difference correlates with duration of deafness rather than age. In contrast to younger adults, significant differences in CI performance are seen in older recipients depending on the side of implantation, with those implanted on the right performing better than those implanted on the left. CONCLUSION: Postlingually deafened older adults obtained significant speech perception benefits from CIs, although differences in speech outcomes were seen between younger recipients and those implanted after age 70. In older adults considering CIs, hearing benefits appear greater if they are implanted in the right ear

This study investigated factors that affect the development of positive peer relationships among deaf children with cochlear implants. Ten 5- to 6-year-old deaf children with implants were observed under conditions varying peer context difficulty in a Peer Entry task. Results revealed better outcomes for deaf children interacting in one-on-one situations compared to interactions including two other hearing children and better performance among girls than boys. In addition, longer duration of implant use and higher self-esteem were associated with better performance on the Peer Task, which was in turn related to parental reports of children's social functioning outside the experimental situation. These findings contribute to the growing literature describing the benefits of cochlear implantation in the areas of communication and socialization, while pointing to interventions that may enhance deaf children's social competence

Objective: To assess the frequency of electrode deactivation over time in post meningitic cochlear implant (CI) recipients Study Design: Retrospective chart review Methods: A retrospective chart review of all post meningitic cochlear implant recipients at New York University from 1984 2008 was conducted Patients with more than 2 years of follow up programming and speech perception data were included. Percent of active electrodes was calculated relative to maximum number of programmable electrodes Frequency and pattern of electrode deactivation over time was analyzed and compared to published data on non meningitic CI patients Results: A total of 14 patients with 17 implanted ears were included. Length of follow up ranged from 2 25 years (average 7.3). A total of 9 (53%) ears experienced a reduction in active electrodes. Of these, 3 patients had deactivation of 1 electrode, 4 patients lost 2 electrodes, and the remaining 2 patients had had 5 and 6 electrodes deactivated, respectively. All patients except one had a minimum of 11 active electrodes at all times. Rate of deactivation over time was variable with loss of electrodes occurring up to 4 years post operatively. Three of the 9 ears with electrode deactivation received Nucleus 24 double array devices Loss of electrodes was not correlated with a decline in speech perception, age at implantation or duration of deafness. There was one device failure. Conclusions: Deactivation of CI electrodes over time is common in post meningitic CI recipients (53%) and exceeds rates from non meningitic patients (1%.). Although electrode deactivation is multi factorial, anatomic considerations, such as ongoing compromise of the electrode neural interface by labyrinthitis ossificans, may contribute to deactivation in both the short and long term

OBJECTIVE: To investigate whether children implanted in the first year of life show higher levels of speech perception than later-implanted children, when compared at the same ages and to investigate the time course of sensitive periods for developing speech perception skills. More specifically, to determine whether faster gains in speech perception are made by children implanted before 1 year old relative to those implanted at 2 or 3 years. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary academic referral center. PATIENTS: 117 children with congenital profound bilateral sensorineural hearing loss, with no additional identified disabilities. INTERVENTION: Cochlear implantation in the first, second, or third year of life. MAIN OUTCOME MEASURE: Development curves showing Lexical Neighborhood Test (LNT) word identification scores as a function of age. RESULTS: Children implanted within the first year of life have a mean advantage of 8.2% LNT-easy word scores over those implanted in the second year (p < 0.001) and a 16.8% advantage in LNT-easy word scores over those implanted in the third year of life (p < 0.001). These advantages remained statistically significant after accounting for sex, residual hearing, and bilateral cochlear implant use. When speech perception scores were expressed as a function of 'hearing age' rather than chronological age, however, there were no significant differences among the 3 groups. CONCLUSION: There is a clear speech perception advantage for earlier-implanted children over later-implanted children when compared at the same age but not when compared at the same time after implantation. Thus, the sensitive period for developing word identification seems to extend at least until age 3 years

OBJECTIVE:: To determine whether intraoperative neural response telemetry (tNRT) is predictive of postoperative speech perception. STUDY DESIGN:: Retrospective review. SETTING:: Tertiary referral center. PATIENTS:: Children (n = 24) aged between 5 and 17 years and adults 18 years and older (n = 73) with severe-to-profound hearing loss and implanted with the Nucleus Freedom device between 2005 and 2008 and observed at least 1 year were included. INTERVENTION:: Intraoperative neural response telemetry after insertion of the electrode array. MAIN OUTCOME MEASURE:: Measures included 1) intraoperative tNRT measurements and 2) preoperative and 1-year postoperative open-set word recognition scores using age-appropriate open-set tests for children and adults. Intraoperative neural response telemetry levels for electrodes E20, E15, E10, and E5 in each patient were correlated to performance at the 1-year evaluation interval. RESULTS:: No correlation existed between tNRT responses and open-set speech performance at the 1-year evaluation. Several patients had absent tNRT in the OR but developed speech recognition abilities, whereas the remaining patients had intraoperative responses with levels of postoperative performance ranging from 0% to 100%. CONCLUSION:: This study suggests that there is no significant correlation between intraoperative tNRT and speech perception performance at 1 year. At the time of surgery, tNRT provides valuable information regarding the electrical output of the implant and the response of the auditory system to electrical stimulation and preliminary device programming data; however, it is not a valuable predictor of postoperative performance. Furthermore, the absence of tNRT does not necessarily indicate a lack of stimulation

OBJECTIVES: To investigate the influence of age, and age-at-implantation, on speech production intelligibility in prelingually deaf pediatric cochlear implant recipients. METHODS: Forty prelingually, profoundly deaf children who received cochlear implants between 8 and 40 months of age. Their age at testing ranged between 2.5 and 18 years. Children were recorded repeating the 10 sentences in the Beginner's Intelligibility Test. These recordings were played back to normal-hearing listeners who were unfamiliar with deaf speech and who were instructed to write down what they heard. They also rated each subject for the intelligibility of their speech production on a 5-point rating-scale. The main outcome measures were the percentage of target words correctly transcribed, and the intelligibility ratings, in both cases averaged across 3 normal-hearing listeners. RESULTS: The data showed a strong effect of age at testing, with older children being more intelligible. This effect was particularly pronounced for children implanted in the first 24 months of life, all of whom had speech production intelligibility scores of 80% or higher when they were tested at age 5.5 years or older. This was true for only 5 out of 9 children implanted at age 25-36 months. CONCLUSIONS: Profoundly deaf children who receive cochlear implants in the first 2 years of life produce highly intelligible speech before the age of 6. This is also true for most, but not all children implanted in their third year

OBJECTIVES/HYPOTHESIS:: Medulloblastoma is the most common pediatric malignant tumor of the central nervous system in children. Treatment includes surgical excision, external beam radiation, and multiagent chemotherapy. Otologic sequelae are common and may result from radiation and/or chemotherapy. Profound sensorineural hearing loss (SNHL) is a known complication of neuro-oncologic treatment and may render these patients eligible for cochlear implantation (CI). Issues of CI in this population, including diagnosis, treatment of preoperative middle ear disease, operative and postoperative course, performance data, and long-term tumor surveillance are highlighted and reviewed. STUDY DESIGN:: Retrospective chart review. METHODS:: Three patients treated for pediatric medulloblastoma with surgical resection, postoperative hyperfractioned craniospinal radiotherapy, and multiagent adjuvant chemotherapy who underwent cochlear implantation were identified. Details of neuro-oncologic treatment and associated otologic complications are presented and analyzed. Primary outcome assessment includes treatment of middle ear pathology, perioperative cochlear implant course, and postimplantation performance data. RESULTS:: Each patient required surgical treatment of chronic ear disease 4 to 16 years after chemoradiation. All progressed to profound SNHL and were implanted 8 to 17 years post-neuro-oncologic treatment. There were no intraoperative complications, and full insertion of the cochlear implant electrode array was achieved in each patient. One patient developed postoperative wound dehiscence requiring operative closure. Postimplantation performance data support significant benefit in all patients. CONCLUSIONS:: Patients treated for pediatric medulloblastoma develop otologic sequelae, including profound SNHL, and may require cochlear implantation. Successful management of middle ear and mastoid pathology involves consideration of potential future cochlear implantation. Postoperative performance data supports cochlear implantation in this population. Laryngoscope, 2009

OBJECTIVES/HYPOTHESIS: Widespread universal newborn hearing screening has led to increased identification of infant hearing loss. Supported by improved diagnostic tools allowing more definitive diagnosis of profound sensorineural hearing loss in young children, cochlear implantation in children <12 months of age is now common. Literature supports short-term safety and improved auditory outcomes in these young children, however long-term data is lacking. The study examines issues of long-term safety and efficacy in cochlear implant patients implanted <1 year of age. STUDY DESIGN: Retrospective chart review. METHODS: Fifty children who received cochlear implants before 1 year of age were followed for up to 7 years. Age at implantation ranged from 5 to 11 months with a mean of 9.1 months. Three patients had simultaneous bilateral implantation at 8 to 9 months of age. Medical records were reviewed for complications incurred during length of device usage, including time of complication, management, and resolution. Auditory assessment included both the Infant-Toddler Meaningful Auditory Integration Scale and tests of speech perception. RESULTS: All 50 patients had full insertions of the electrode array. There were a total of eight complications (16%) in seven patients, three major (6%) and five minor (10%), which occurred at or before 10 months postoperatively. There were no perioperative anesthetic complications. CONCLUSIONS: Cochlear implantation in children <12 months of age is safe and efficacious over an extended period of time. Rates and nature of both major and minor complications are comparable to studies in adults and older children and support continued monitoring of these patients over the long-term